ICD-10-CM Code E80.29

Other porphyria

Version 2020 Billable Code

Valid for Submission

E80.29 is a billable code used to specify a medical diagnosis of other porphyria. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code E80.29 might also be used to specify conditions or terms like adverse effect from puva photochemotherapy, chester-type porphyria, congenital porphyria, coproporphyria, coproporphyrinuria, drug-induced porphyria, etc

Short Description:Other porphyria
Long Description:Other porphyria

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code E80.29:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Hereditary coproporphyria

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E80.29 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Adverse effect from PUVA photochemotherapy
  • Chester-type porphyria
  • Congenital porphyria
  • Coproporphyria
  • Coproporphyrinuria
  • Drug-induced porphyria
  • Drug-induced pseudoporphyria
  • Erythropoietic coproporphyria
  • Ferrochelatase deficiency
  • Hemodialysis-associated pseudoporphyria
  • Hepatic porphyria
  • Hereditary coproporphyria
  • Homozygous hereditary coproporphyria
  • Inherited disorder of porphyrin metabolism
  • Inherited disorder of porphyrin metabolism
  • Porphobilinogen deaminase deficiency
  • Porphobilinogen synthase deficiency
  • Porphyria-like reaction to poison and/or environmental toxin
  • Porphyruria
  • Porphyruria
  • Protoporphyrinuria
  • Pseudoporphyria
  • Pseudoporphyria
  • Pseudoporphyria
  • Pseudoporphyria
  • Pseudoporphyria
  • Pseudoporphyria due to PUVA therapy
  • Skin lesion associated with hemodialysis
  • Toxicoderma
  • Uroporphyrinogen decarboxylase deficiency
  • Uroporphyrinuria

Clinical Information

  • PORPHYRIAS-. a diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of heme in the liver the bone marrow or both. they are classified by the deficiency of specific enzymes the tissue site of enzyme defect or the clinical features that include neurological acute or cutaneous skin lesions. porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.
  • PORPHYRIA ERYTHROPOIETIC-. an autosomal recessive porphyria that is due to a deficiency of uroporphyrinogen iii synthase in the bone marrow; also known as congenital erythropoietic porphyria. this disease is characterized by splenomegaly; anemia; photosensitivity; cutaneous lesions; accumulation of hydroxymethylbilane; and increased excretion of uroporphyrins and coproporphyrins.
  • PORPHYRIAS HEPATIC-. a group of metabolic diseases due to deficiency of one of a number of liver enzymes in the biosynthetic pathway of heme. they are characterized by the accumulation and increased excretion of porphyrins or its precursors. clinical features include neurological symptoms porphyria acute intermittent cutaneous lesions due to photosensitivity porphyria cutanea tarda or both hereditary coproporphyria. hepatic porphyrias can be hereditary or acquired as a result of toxicity to the hepatic tissues.
  • PORPHYRIA ACUTE INTERMITTENT-. an autosomal dominant porphyria that is due to a deficiency of hydroxymethylbilane synthase in the liver the third enzyme in the 8 enzyme biosynthetic pathway of heme. clinical features are recurrent and life threatening neurologic disturbances abdominal pain and elevated level of aminolevulinic acid and porphobilinogen in the urine.
  • PORPHYRIA CUTANEA TARDA-. an autosomal dominant or acquired porphyria due to a deficiency of uroporphyrinogen decarboxylase in the liver. it is characterized by photosensitivity and cutaneous lesions with little or no neurologic symptoms. type i is the acquired form and is strongly associated with liver diseases and hepatic toxicities caused by alcohol or estrogenic steroids. type ii is the familial form.
  • PORPHYRIA HEPATOERYTHROPOIETIC-. an autosomal recessive cutaneous porphyria that is due to a deficiency of uroporphyrinogen decarboxylase in both the liver and the bone marrow. similar to porphyria cutanea tarda this disorder is caused by defects in the fifth enzyme in the 8 enzyme biosynthetic pathway of heme but is a homozygous enzyme deficiency with less than 10% of the normal enzyme activity. cutaneous lesions are severe and mutilating.
  • PORPHYRIA VARIEGATE-. an autosomal dominant porphyria that is due to a deficiency of protoporphyrinogen oxidase ec in the liver the seventh enzyme in the 8 enzyme biosynthetic pathway of heme. clinical features include both neurological symptoms and cutaneous lesions. patients excrete increased levels of porphyrin precursors coproporphyrins and protoporphyrinogen.

Convert E80.29 to ICD-9

  • 277.1 - Dis porphyrin metabolism (Approximate Flag)

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Disorders of porphyrin and bilirubin metabolism (E80)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients


Porphyrias are a group of genetic disorders caused by problems with how your body makes a substance called heme. Heme is found throughout the body, especially in your blood and bone marrow, where it carries oxygen.

There are two main types of porphyrias. One affects the skin and the other affects the nervous system. People with the skin type develop blisters, itching, and swelling of their skin when it is exposed to sunlight. The nervous system type is called acute porphyria. Symptoms include pain in the chest, abdomen, limbs, or back; muscle numbness, tingling, paralysis, or cramping; vomiting; constipation; and personality changes or mental disorders. These symptoms come and go.

Certain triggers can cause an attack, including some medicines, smoking, drinking alcohol, infections, stress, and sun exposure. Attacks develop over hours or days. They can last for days or weeks.

Porphyria can be hard to diagnose. It requires blood, urine, and stool tests. Each type of porphyria is treated differently. Treatment may involve avoiding triggers, receiving heme through a vein, taking medicines to relieve symptoms, or having blood drawn to reduce iron in the body. People who have severe attacks may need to be hospitalized.

NIH: National Institute of Diabetes and Digestive and Kidney Diseases

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Porphyria Reviews porphyria, a group of disorders that affect the skin, nervous system, or both. Discusses types, causes, symptoms, diagnosis, and treatment.
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Porphyria Porphyria is a group of disorders caused by abnormalities in the chemical steps that lead to heme production. Heme is a vital molecule for all of the body's organs, although it is most abundant in the blood, bone marrow, and liver. Heme is a component of several iron-containing proteins called hemoproteins, including hemoglobin (the protein that carries oxygen in the blood).Researchers have identified several types of porphyria, which are distinguished by their genetic cause and their signs and symptoms. Some types of porphyria, called cutaneous porphyrias, primarily affect the skin. Areas of skin exposed to the sun become fragile and blistered, which can lead to infection, scarring, changes in skin coloring (pigmentation), and increased hair growth. Cutaneous porphyrias include congenital erythropoietic porphyria, erythropoietic protoporphyria, hepatoerythropoietic porphyria, and porphyria cutanea tarda.Other types of porphyria, called acute porphyrias, primarily affect the nervous system. These disorders are described as "acute" because their signs and symptoms appear quickly and usually last a short time. Episodes of acute porphyria can cause abdominal pain, vomiting, constipation, and diarrhea. During an episode, a person may also experience muscle weakness, seizures, fever, and mental changes such as anxiety and hallucinations. These signs and symptoms can be life-threatening, especially if the muscles that control breathing become paralyzed. Acute porphyrias include acute intermittent porphyria and ALAD deficiency porphyria. Two other forms of porphyria, hereditary coproporphyria and variegate porphyria, can have both acute and cutaneous symptoms.The porphyrias can also be split into erythropoietic and hepatic types, depending on where damaging compounds called porphyrins and porphyrin precursors first build up in the body. In erythropoietic porphyrias, these compounds originate in the bone marrow. Erythropoietic porphyrias include erythropoietic protoporphyria and congenital erythropoietic porphyria. Health problems associated with erythropoietic porphyrias include a low number of red blood cells (anemia) and enlargement of the spleen (splenomegaly). The other types of porphyrias are considered hepatic porphyrias. In these disorders, porphyrins and porphyrin precursors originate primarily in the liver, leading to abnormal liver function and an increased risk of developing liver cancer.Environmental factors can strongly influence the occurrence and severity of signs and symptoms of porphyria. Alcohol, smoking, certain drugs, hormones, other illnesses, stress, and dieting or periods without food (fasting) can all trigger the signs and symptoms of some forms of the disorder. Additionally, exposure to sunlight worsens the skin damage in people with cutaneous porphyrias.
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